Malignant schwannomas of the oral cavity and especially inferior dental nerve tumours, are rare malignancies. Mostly arising de novo as solitary tumours, some cases have occurred in Von Recklinghausen neurofibromatosis (25% of cases) or, as in the present report, at the time of a relapse by transformation of a benign schwannoma. Long survival is only observed in selected early diagnosed patients additionally treated by extended surgery. Support of magnetic resonance imaging and immunohistological techniques in diagnosis are stressed. Advances in reconstructive surgical devices after debulking surgery have contributed to enhance the performance of more curative procedures (hemimandibulectomy, intra oral resections) and to improve the functional and esthetic results in these bad prognosis nerve tumours of the oral cavity.